title: Clinical characteristics and outcome of isolated extracerebral relapses of primary central nervous system lymphoma: a case series.
abstract: There is very limited data on isolated systemic relapses of primary central nervous system lymphomas (PCNSL). We retrospectively reviewed the clinical characteristics and outcome of 10 patients with isolated systemic disease among 209 patients with PCNSL mainly treated with methotrexate-based chemotherapy (CT) with or without radiation therapy (RT). Isolated systemic relapse remained rare (4.8%, 10/209 patients). Median time from initial diagnosis to relapse was 33 months (range, 3-94). Sites of relapse were mostly extranodal. Three patients presented with early extra-cerebral (EC) relapse 3, 5 and 8 months from the beginning of initial treatment, respectively, and 7 patients had later relapses (range, 17-94 months). Treatment at relapse included surgery alone, RT alone, CT with or without radiotherapy, or CT with autologous stem cell transplantation (ASCT). Median overall survival (OS) after relapse was 15.5 months (range, 5.8-24.5) compared to 4.6 months (range, 3.6-6.5) for patients with central nervous system (CNS) relapse (p = 0.35). In conclusion, isolated systemic relapses exist but are infrequent. Early EC relapse suggests the presence of systemic disease undetectable by conventional evaluation at initial diagnosis. Patient follow-up must be prolonged because systemic relapse can occur as late as 10 years after initial diagnosis. Whether EC relapses of PCNSL have a better prognosis than CNS relapses needs to be assessed in a larger cohort.
authors.affiliation: Department of Medical Oncology, Centre Léon Bérard, Université de Lyon, Lyon, France.
authors.forename: Sawyna
authors.initials: S
authors.lastname: Provencher
authors.forename: Céline
authors.initials: C
authors.lastname: Ferlay
authors.forename: Khaoula
authors.initials: K
authors.lastname: Alaoui-Slimani
authors.forename: Alain
authors.initials: A
authors.lastname: Devidas
authors.forename: Stephane
authors.initials: S
authors.lastname: Lepretre
authors.forename: Bernard
authors.initials: B
authors.lastname: de Prijck
authors.forename: Catherine
authors.initials: C
authors.lastname: Sebban
authors.forename: Arnaud
authors.initials: A
authors.lastname: de la Fouchardiere
authors.forename: Catherine
authors.initials: C
authors.lastname: Chassagne-Clement
authors.forename: Nicolas
authors.initials: N
authors.lastname: Ketterer
authors.forename: Antoine
authors.initials: A
authors.lastname: Thyss
authors.forename: André
authors.initials: A
authors.lastname: Delannoy
authors.forename: Hervé
authors.initials: H
authors.lastname: Tilly
authors.forename: Pierre
authors.initials: P
authors.lastname: Biron
authors.forename: Jean-Yves
authors.initials: JY
authors.lastname: Blay
authors.forename: Hervé
authors.initials: H
authors.lastname: Ghesquières
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D016543:Central Nervous System Neoplasms / Q000401:mortality / Q000628:therapy*; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D008228:Lymphoma, Non-Hodgkin / Q000401:mortality / Q000628:therapy*; D008297:Male; D008727:Methotrexate / Q000627:therapeutic use; D008875:Middle Aged; D012008:Recurrence; D016896:Treatment Outcome
chemical_list: D008727:Methotrexate